Macroglossia Secondary to Primary Amyloidosis of the Tongue

Abstract

ABSTRACTIntroductionAmyloidosis is a disease entity characterized by the presence of proteinaceous material deposited extracellularly at various locations in the body and in the head and neck region.Materials and methodsWe present a case of macroglossia secondary to amyloidosis of the tongue in a 65-year-old male. He presented with gradually progressive diffuse enlargement of the tongue, difficulty in speech and progressive difficulty in breathing while sleeping. Patient was managed conservatively with steroids and alkylating agents.DiscussionThe most common sites of involvement in systemic amyloidosis are the tongue (63%) and the larynx (19%). Although lingual involvement is common, macroglossia as a presenting symptom, though pathognomonic is rare (5%). Amyloidosis of the tongue typically results in macroglossia, manifested by increased tongue volume, tongue protrusion beyond the alveolar ridge, speech impairment, drooling and dysphagia.ConclusionAmyloidosis of the tongue is almost always secondary to systemic disease. An extensive workup is required to differentiate between systemic and localized amyloidosis. The effect of treatment is difficult to estimate, and further research needs to be focused on this aspect. These obstacles can make the diagnosis and management of these lesions particularly challenging.